演題詳細

ポスター / Poster

ポスター 39 (Poster 39) :後天性血友病

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日程
2013年10月11日(金)
時間
16:50 - 17:50
会場
ポスター会場 / Poster (ロイトン札幌 3F ロイトンホールABCD)
座長・司会
森下 英理子 (Eriko Morishita):1
1:金沢大学医薬保健研究域病態検査学
 
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Successful treatment with rituximab in a case of coincidence of TTP and acquired hemophilia A

演題番号 : PS-1-296

三道 康永 (Yasuhisa Sando):1、山本 和彦 (Kazuhiko Yamamoto):1、石川 立則 (Tatsunori Ishikawa):1、新谷 勝美 (Masami Niiya):2、逸見 麻衣 (Mai Henmi):1、清家 圭介 (Keisuke Seike):1、原 嘉孝 (Yoshitaka Hara):1、牧田 雅典 (Masanori Makita):1、今城 健二 (Kenji Imajo):1

1:Okayama City Hospital, Department of Hematology/Oncology、2:Niiya Family Practitioner

 

TTP and Acquired hemophilia A (AHA) are known as the rare life-threatening illness, which resulted from the development of specific inhibitor anti ADAMTS13 and FVIII. Herein we report a case of concurrent expression of these distinct inhibitors, which have achieved remission by introducing rituximab. In September 2011, a 65-year-old man was admitted because of purpura and prolongation of APTT. FVIII activity decreased to <1% and developed FVIII inhibitor (14BU/ml). He was diagnosed as AHA and medicated with PSL. APTT was gradually normalized and PSL was tapered. In April 2012, Factor VIII inhibitor titer rose again. He was readmitted and found thrombocytopenia and autoimmune hemolytic anemia. We speculated Evans syndrome accompanied with AHA, and treated with IVIG. But the platelet count was not increased. Speech disturbance and delirium were observed unstably from the 12th hospital day. Then he was diagnosed as TTP, because ADAMTS13 inhibitor (1.4BU/ml) was detected, and resulted in the decline of ADAMTS13 activity (<0.5%). We initiated the plasma exchange (PE) and steroid-pulse therapy on the day24. After having provided PE for 3 days, lab data and the psychiatric symptoms were improved dramatically. However, after 2-day intermission of PE, platelet count decreased markedly. Therefore, we applied rituximab to eradicate these inhibitors on the day33. Then the platelet count was recovered quickly and he could get withdrawal from PE. After additional twice administration of rituximab, both inhibitors had not been detected, and maintain complete remission from then onward.

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